CLINICAL SYMPTOMS AT DIFFERENT AGES IN AUTOSOMAL-DOMINANT RETINITIS-PIGMENTOSA - A FAMILY STUDY IN 3 GENERATIONS

A family with autosomal dominant retinitis pigmentosa in three generat ions. The age of the patients varied from 11 to 66 years. From the you ngest to the eldest member of the family, the visual acuity varied fro m 1.O to 0.4, the defect in the visual fields from a relative ring sco toma to a tubular field of 5 degrees, and the rod threshold in dark ad aptation from normal to an elevation of 3 log units. Color vision was normal in all of the family members. The fundus changes varied from co arseness of retinal pigment epithelium to bone spicule pigmentation wi th narrow vessels and pale optic nerve head. The progression of autoso mal dominant retinitis pigmentosa has been reported to be milder than in the other types of retinitis pigmentosa. The clinical symptoms of t he family in the present study confirm this finding.